Postinfantile giant-cell hepatitis associated with ulcerative colitis and autoimmune hepatitis

Giant cell hepatitis (GCH) is commonly reported in neonatal and infantile liver diseases but rarely in adults where the term postinfantile GCH (PIGCH) is used. PIGCH is associated with many diseases, including drugs toxicity, viruses, and autoimmune liver diseases, with autoimmune hepatitis (AIH) being the most prevalent. We present a case of PIGCH in a 76-year-old female without known history of liver disease who suffered from an acute severe episode of hepatitis. After careful exclusion of other hepatitis causes by imaging, virological, immunological, and microbiological investigations, a diagnosis of acute severe AIH (AS-AIH) was established. The patient was started on corticosteroids but she did not respond and died 3 days later because of advanced acute liver failure. Postmortem liver biopsy showed typical PIGCH lesions. Physicians must keep this catastrophic entity in mind in cases of unexplained acute liver injury as, contrary to our case, prompt rescue therapy with corticosteroids may be life-saving.

Download Full-text

Is simultaneous presence of IgG4-positive plasma cells and giant-cell hepatitis a coincidence in autoimmune hepatitis? A case report

World Journal of Clinical Cases ◽

10.12998/wjcc.v9.i25.7527 ◽

2021 ◽

Vol 9 (25) ◽

pp. 7527-7534

Author(s):

You-Wen Tan ◽

Jia-Min Wang ◽

Li Chen

Keyword(s):

Case Report ◽

Autoimmune Hepatitis ◽

Giant Cell ◽

Hepatitis A ◽

Plasma Cells ◽

Simultaneous Presence ◽

Giant Cell Hepatitis

Download Full-text

Postinfantile giant cell hepatitis in the setting of autoimmune hepatitis: exclusively a histological pattern or a prognosis predictor?

BMJ Case Reports ◽

10.1136/bcr-2021-243660 ◽

2021 ◽

Vol 14 (7) ◽

pp. e243660

Author(s):

Carolina Teles ◽

Rui Santos ◽

Carlos Dias Silva ◽

Teresa Vaio

Keyword(s):

Liver Disease ◽

Liver Failure ◽

Autoimmune Hepatitis ◽

Immunosuppressive Therapy ◽

Giant Cell ◽

Viral Infections ◽

Chronic Liver ◽

Close Monitoring ◽

Histological Pattern ◽

Giant Cell Hepatitis

Autoimmune hepatitis (AIH) is a rare chronic liver disease with a non-specific clinical presentation. Its physiopathology is not fully understood and, if untreated, can progress to cirrhosis and even fulminant liver failure. Here, we describe a case of a 73-year-old patient with an 11-month history suggestive of liver disease, who was concomitantly diagnosed with AIH and the extremely rare postinfantile giant cell hepatitis (PIGCH). Despite standard immunosuppressive therapy, the patient presented a severe clinical course, culminating in acute-on-chronic liver failure and death. This case reminds physicians of the importance of an early diagnosis, close monitoring and timely treatment of AIH. It also highlights the significant role in prognosis of the specific histological pattern of PIGCH, which has been mainly associated with a serious clinical outcome and unpredictable response to immunosuppressive therapy. Triggers of both AIH and PIGCH, such as viral infections, must be excluded, given their treatment implications.

Download Full-text